Madeleine Hull
Cystic Fibrosis (CF) is a life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract, making it difficult for patients to breathe and digest food. As a result, people with CF often require a higher calorie intake to maintain a healthy weight. The recommended daily calorie intake for CF patients is up to twice that of individuals without CF. This can be achieved through a well-balanced diet that includes high-calorie, high-protein, and high-fat foods. In addition to dietary adjustments, CF patients may also require pancreatic enzyme replacements and vitamin and mineral supplements to aid digestion and absorption of nutrients.
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What You'll Learn
High-calorie, high-fat foods
People with cystic fibrosis (CF) may require a high-calorie, high-fat diet to meet their increased energy needs, which can be 1.5 to 2 times higher than those without CF. This is because the mucus in CF patients can block the ducts in the pancreas, affecting the production of digestive enzymes and leading to difficulty in properly absorbing nutrients.
A dietitian can suggest ways to add calories to your diet and recommend nutritional supplements if needed. It is important to note that these supplements should be taken alongside meals, not as a replacement. Additionally, CF patients should aim for a balanced diet that incorporates unsaturated fats found in foods like avocados, nuts, and oils, as a purely saturated fat-based diet can increase the risk of heart disease.
Some high-calorie, high-fat food options that can help meet energy needs include:
- Grilled ham and cheese sandwich with avocado
- Spaghetti with extra cheese and olive oil
- Peanut butter and jelly sandwich with a glass of whole milk
- Nuts or trail mix
- Full-fat yoghurt with granola
- Cheese crackers
- Whole-milk cottage cheese
- Hummus with pita bread
- Bean, cheese, and salsa burrito
- Grilled chicken Caesar wrap
It is also recommended to carry easy-access snacks, such as cheese sticks, single-serving fruits with heavy syrup, crackers, nuts, dried fruit, and bottled coffee drinks.
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Salt and fluid intake
People with cystic fibrosis (CF) should follow a balanced diet with a particular focus on calorie and protein intake. This is because the condition causes a build-up of mucus in the lungs and digestive tract, making it difficult to digest food and absorb nutrients.
CF patients tend to lose more salt in their sweat than others, so it is important to replenish this through their diet. Dietitians may recommend adding extra salt to meals, eating salty snacks, and drinking sports drinks when exercising or spending time outdoors in hot weather. People with CF who live in hot climates may need to increase their salt intake.
It is important for CF patients to stay hydrated, as every cell in the body needs water to function properly. The amount of fluid a person needs depends on their age, weight, activity level, general health, and the climate. It is recommended to drink water regularly throughout the day and to monitor urine colour, which should be clear or a pale yellow. Waiting until you feel thirsty to drink water may mean that you are already dehydrated. Symptoms of dehydration include headaches, nausea, loss of appetite, and fatigue.
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Weight gain and BMI
Weight management is a critical aspect of cystic fibrosis (CF) care, as the condition affects the body's ability to digest food and absorb nutrients. People with CF often require up to twice the daily calories of their peers to maintain their weight, and even more to gain weight. The goal for weight management in CF is typically sustained weight gain and growth in children and teens, and weight maintenance for adults.
Body Mass Index (BMI) is a commonly used metric to assess whether an individual is at a healthy weight relative to their height. However, the desired BMI can vary depending on age and height. For instance, babies and preschoolers with CF should have a BMI at or above the 50th percentile for their height, which may result in a slightly chubby appearance. This higher height-to-weight ratio is linked to better lung function later in life. For children with CF between the ages of 2 and 20, the goal is typically to maintain a BMI at or above the 50th percentile for their height. The Cystic Fibrosis Foundation recommends that adult women maintain a BMI of at least 22, while adult men should aim for a BMI of at least 23, as these levels are associated with optimal lung function.
Achieving and maintaining a healthy weight can be challenging for individuals with CF. They may require additional calories and nutritional support to meet their weight goals. A CF diet typically includes high-calorie, high-fat, and high-salt foods, along with fruits and vegetables. A diet with 40% of total calories from fat is commonly recommended. However, it is important to incorporate unsaturated fats, such as avocados, nuts, and oils, to reduce the risk of heart disease. Nutritional supplements, in the form of flavoured powders, milk-based shakes, and high-calorie juices, can also aid in weight gain.
In some cases, individuals with CF may require more aggressive interventions to achieve weight gain. Tube feedings, also known as enteral nutrition, can provide overnight feedings to boost nutritional intake. While this method can be effective, it may be costly, leave scars, and impact self-image. It is important to work closely with a CF dietitian to determine the best approach to weight management, as they can provide tips and ideas to ensure adequate nutritional intake without causing eating fatigue.
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Supplements and vitamins
Cystic fibrosis (CF) is a disease that causes thick, sticky mucus to build up in the lungs and digestive tract, making it difficult for people with CF to digest food and absorb nutrients. As a result, people with CF often require a high-calorie, high-protein, and high-fat diet to maintain a healthy weight and meet their nutritional needs.
People with CF may require additional supplements and vitamins to ensure they are getting adequate nutrition. Firstly, most people with CF need to take pancreatic enzymes to help their bodies absorb fat and protein. These enzymes can help reduce foul-smelling stools, gas, and bloating. Additionally, fat-soluble vitamins A, D, E, and K are often recommended for people with CF, as they are important for immune function, growth, and healing. These vitamins are absorbed along with fat, and CF patients may have trouble absorbing them due to difficulties in digesting and absorbing fat. Calcium supplements are also recommended for CF patients, especially those with pancreatic insufficiency, as they are at risk for osteopenia or osteoporosis (weak, brittle bones).
Furthermore, individuals with CF may benefit from adding unsaturated fats to their diet through foods such as avocados, nuts, and oils like walnut and flaxseed oils. This is because a 2012 study found an overdependence on saturated fats and a low intake of polyunsaturated fats in CF patients, which can increase the risk of heart disease over time.
High-calorie supplements can be used to boost calorie intake, but it is important that they do not replace meals. Instead, they should be taken alongside meals or as a substitute for lower-calorie snacks. Flavoured powders that can be mixed with milk, ready-made milk-based shakes, and high-calorie, high-protein juices are some options available. It is important to consult with a dietitian to determine the right choices and quantities for your individual needs.
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Nutritional snacks
People with CF need to eat foods that are high in calories and protein throughout the day. The energy needs of people with CF are estimated to be 1 1/2 to 2 times the needs of those without CF. A high-calorie, high-fat diet, with 40% of total calories from fat is generally recommended.
- Cheese and crackers are a great source of protein and fat.
- Muffins can be a good snack option, especially when paired with a protein source such as peanut butter.
- Trail mix is a portable and nutritious snack that provides protein, healthy fats, and carbohydrates.
- Full-fat yoghurt with granola is a delicious and creamy snack that provides protein, calcium, and healthy fats.
- Nuts are a good source of protein and healthy fats, and can be easily incorporated into trail mix or eaten on their own.
- Peanut butter and banana sandwiches are a tasty and calorie-dense option.
- Nutritional supplement milkshakes are a convenient way to boost calories and protein intake.
- Fruits such as bananas provide natural sugars and essential vitamins.
- Vegetables with dip such as peanut butter, hummus, or sour cream can increase your vegetable intake.
- Meat, chicken, and fish can be a good source of protein when paired with other snacks.
It is important to work with a specialist dietitian who can advise on an individual's nutritional requirements and help them reach their BMI goal.
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Frequently asked questions
CF patients should follow a diet that is high in calories and protein. This is because people with CF need up to twice the amount of daily calories as others of a similar age and weight. It is also important to eat a well-balanced diet, with a focus on whole grains, fruits and vegetables, and healthy fats.
There are several ways to add extra calories to the diet of a CF patient:
- Add extra butter or oil to pasta, rice, potatoes, or cooked vegetables.
- Pair raw vegetables with salad dressing or hummus.
- Top salads and sandwiches with avocado or guacamole.
- Grill sandwiches in butter or margarine.
- Add a splash of heavy cream to milk, oatmeal, smoothies, or cream-based soups.
- Focus on high-calorie snacks such as trail mix, peanut butter and banana sandwiches, full-fat yoghurt with granola, or cheese crackers.
It is important to consider the specific needs of the individual when planning the diet of a CF patient. Age, weight, height, symptoms, lung function, and activity level will all impact the dietary and nutritional requirements of the patient. It is also important to work with a doctor or dietitian to ensure the patient is getting the right balance of nutrients and calories.
